Primary Intracranial Squamous Cell Carcinoma

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1 Neurol Med Chir (Tokyo) 39, 49 `54, 1999 Primary Intracranial Squamous Cell Carcinoma -Case Report- Satoru MURASE, Hiroyasu YAMAKAWA, Akio OHKUMA* *, Yasuhiko SUMI * * *, Masaharu KAJIWARA*, Tsuyoshi TAKAMI*, and Noboru SAKAI Department of Neurosurgery and *Second Department of Pathology, Gifu University School of Medicine, Gifu; **Department of Neurosurgery, Prefectural Gifu Hospital, Gifu; ***Department of Neurosurgery, Hakuhou-kai Sumi Hospital, Shirotori, Gifu Abstract A 50-year-old female presented with primary intracranial squamous cell carcinoma (SCC) at the right cerebellopontine angle manifesting as right facial nerve paresis. She had undergone gross total removal of a right cerebellopontine angle epidermoid cyst 10 years before and had done well until recently. Magnetic resonance imaging showed a heterogeneous tumor with markedly enhanced irregular margin. Subtotal removal of the tumor was achieved. Histological examination showed moderately differentiated SCC. After surgery, she underwent chemotherapy and gamma radiosurgery. She is now well 5 years after the diagnosis of SCC. Key words: epidermoid cyst, squamous cell carcinoma, brain tumor Introduction Primary intracranial squamous cell carcinoma (SCC) is an extremely rare neoplasm, and it may be associated with benign intracranial epidermoid or dermoid cyst. We report a case of primary intracranial SCC in a 50-year-old female with a history of subtotal removal of typical epidermoid cyst at the age of 39 years. The tumor was successfully treated with surgical removal followed by chemotherapy and gamma radiosurgery. cranial nerves. Histological examination of the tissue specimen showed benign epidermoid cyst (Fig. 1). Postoperatively, the patient had no deficits and free from the abnormal facial movement at discharge. Second admission: She was well until February 1993, when she noticed drooling from her mouth on Case Report First admission: A 39-year-old female presented with a 2-year history of facial involuntary movement on July 3, On admission, neurological examination revealed hemifacial spasm and mild hearing disturbance on the right side. Computed tomography demonstrated a nonenhancing mass at the right cerebellopontine cistern. Surgery via a right suboccipital craniectomy exposed a typical epidermoid cyst. The tumor capsule was opened and the intracapsular contents were totally removed, but a small part of the capsule could not be removed because of tight adherence to the seventh and eighth Received June 5, 1998; Accepted September 8, 1998 Fig. 1 Photomicrograph of the cyst wall at the initial operation showing flattened and regular squamous epithelium and keratinized squames. HE stain, x ~30. 49

2 S. Murase et al. the right side and asymmetry of her face. On admission, neurological examination revealed seventh cranial nerve paresis and mild hearing disturbance on the right side. Magnetic resonance (MR) imaging suggested recurrence of the previous tumor. The tumor was mainly seated intraaxially in the right cerebellar hemisphere, and a part of the tumor was, exposed to the right cerebellopontine cistern (Fig. 2) Angiography showed that the lesion was avascula] (not shown). At the second operation, the righ cerebellopontine cistern was fully occupied by dry cheesy, yellowish contents which had prolapsec from the thick and grayish capsule. The intraopera tive findings revealed that the tumor was not as pearly as at the initial operation. The contents were dissected free and sucked out as much as possible However, a part of the tumor had invaded into the brain stem with tight adherence, so total removal was impossible. Histological examination of the ex. cised tissue revealed moderately differentiated SCC (Fig. 3). Fig. 2 T1,-weighted magnetic resonance image at the second admission demonstrating a heterogeneous recurrent tumor with marked enhancement of the irregular margin by gadolinium-diethylenetri aminepenta-acetic acid. The tumor is mainly seated intraaxially in the right cerebellar hemisphere, and a part of the tumor is exposed to the right cerebellopontine cistern. Fig. 3 Low-power view of the cyst wall at the second operation showing thickened wall and hypercellularity. There is no necrotic lesion nor dermoid components such as fatty tissue, sebaceous gland, eccrine gland or hair follicle (left: HE stain, ~10). High-power view of the cyst wall showing nuclear and cellular pleomorphism, nuclear enlargement, hyperchromatism, and mitotic figures. A part of the tissue represents foci of acidophilic keratinous differentiation, however keratin pearl formation is not noted in area of squamous differentiation, indicating moderately differentiated squamous cell carcinoma (right: HE stain, ~ 60). Neurol Med Chir (Tokyo) 39, January, 1999

3 Intracranial SCC 51 radiosurgery for the residual tumor with a marginal dose of 14 Gy (Fig. 4). At discharge, she had right facial nerve paresis and hearing loss. Follow-up MR imaging at 5 years showed no progression of the tumor (Fig. 5). She remains functional and ambulatory 5 years after the diagnosis of intracranial SCC. Discussion Fig. 4 T1-weighted magnetic resonance images at gamma radiosurgery. Fig. 5 T1-weighted magnetic resonance image with gadolinium-diethylenetriaminepenta-acetic acid obtained 5 year after the diagnosis of squamous cell carcinoma demonstrating no recurrence of the tumor. An intensive search revealed no primary carcinoma outside the central nervous system. Tumor markers (carcinoembryonic antigen and SCC) in the serum were negative. Three weeks after the second operation, she underwent three courses of chemotherapy with VMP-F (1 mg of vincristine sulfate, 15 mg of methotrexate, 20 mg of peplomycin sulfate, and 24 mg of calcium folinate), a common chemotherapy for cutaneous SCC, and 10 months after the second operation, she underwent gamma Primary intracranial SCC has been reported in 30 cases (Table 1).1-13, ,31) The patients were aged from 4 to 74 years, but most of them (74%) were in the fifth or sixth decades. There was a male predominance of 2 to 1. The most common location of the origin was cerebellopontine angle and most cases arose from pre-existing benign epidermoid or dermoid cysts. Ten of the 31 cases, including the present case, were diagnosed as benign tumor before the development of SCC, with an interval between the first diagnosis and detection of malignancy from 3 months to 33 years (mean 6.7 years). Chronic inflammatory stimulation due to repeated cyst rupture and subtotal resection of the cyst wall may contribute to the malignant degeneration.1) The symptoms of intracranial SCC vary and are similar to those of benign cerebellopontine angle tumor cases. However, the clinical characteristics of intracranial SCC is very rapid deterioration in the symptoms. The MR imaging appearance of benign epidermoid cysts is well documented.14,29) The generally accepted image pattern is low signal intensity on T1-weighted imaging and high intensity on T2- weighted imaging with a smooth and clear margin, and no enhancement with contrast medium. In contrast, intracranial SCC has an irregular margin with enhancement effect. 16,21,22) The intraoperative features of primary intracranial SCC are a grayish tumor with thickened wall and/or solid part, and unclear margin, which indicates tumor infiltration into the surrounding structures. Benign epidermoid cyst enlarges with desquamation of normal cells into the cyst cavity,1) so the cyst wall appears to be thin and flattened. In contrast, SCC enlarges with progressive cell division, so the cyst wall becomes thick and involves a solid part which might correspond to the enhanced region on MR imaging.22) Most previously reported cases resulted in death within 12 months after the onset of symptoms or establishment of the diagnosis of primary intracranial SCC. Only a few patients were alive over 2 years after the diagnosis. All survivors received radiation therapy. Recently, the usefulness of gam- Neurol Med Chir (Tokyo) 39, January, 1999

4 52 S. Murase et al. Neural Med Chir (Tokyo) 39, January, 1999

5 Intracranial SCC 53 ma radiosurgery was emphasized.") In our case, VMP-F therapy and gamma radiosurgery appeared to be effective for SCC and no particular side effects were recognized. Follow-up MR imaging has detected no recurrence for 5 years and she remains functional. The combination of VMP-F chemotherapy and gamma radiosurgery might be more beneficial for primary intracranial SCC. References 1) Abramson RC, Morawetz RB, Schlitt M: Multiple complications from an intracranial epidermoid cyst: Case report and literature review. Neurosurgery 24: ,1989 2) Akachi K, Takahashi H, Ishijima B, Nakamura Y, Oda M, Takizawa T, Iwamoto M, Kuriyama G, Shizuki K: [Malignant changes in a craniopharyngioma]. No Shinkei Geka 15: , 1987 (Jpn, with Eng abstract) 3) Bondeson L, Falt K: Primary intracranial epidermoid carcinoma. Acta Cytol 28: , ) Dubois PJ, Sage M, Luther JS, Burger PC, Heinz ER, Drayer BP: Malignant change in an intracranial epidermoid cyst: Case report. J Comput Assist Tomogr 5: , ) Ebisudani D, Hamazaki F, Oka H, Okada J, Ohshima T, Matsumoto K, Izumi K: [An autopsy case of primary intracranial squamous cell carcinoma]. No Shinkei Geka 18: , 1990 (Jpn, with Eng abstract) 6) Ernst P: Haufung dysontogenetischer Bildungen am Zentralnervensystem. Verh Dtsch Pathol Ges 15: ,1912 7) Fox E, South EA: Squamous cell carcinoma developing in a intracranial epidermoid cyst (cholesteatoma). I Neurol Neurosurg Psychiatry 28: , ) Garcia CA, McGarry PA, Rodriguez F: Primary intracranial squamous cell carcinoma of the right cerebellopontine angle. J Neurosurg 54: , ) Gi H, Yoshizumi H, Nagao S, Nishioka T, Uno J, Fujita Y: [C-P angle epidermoid carcinoma: A case report]. No Shinkei Geka 18: , 1990 (Jpn, with Eng abstract) 10) Giangaspero F, Manetto V, Ferracini R, Piazza G: Squamous cell carcinoma of the brain with sarcoma-like stroma. Virchows Arch A Pathol Anat Histopathol 402: , ) Gluszcz A: A cancer arising in a dermoid of the brain: A case report. J Neuropathol Exp Neurol 21: , ) Goldman SA, Gandy SE: Squamous cell carcinoma as a late complication of intracerebroventricular epidermoid cyst. J Neurosurg 66: , ) Henkel HG: Bensondere Forman bosartiger Erkrankungen der weiched Hirnhaute. Verh Dtsch Ges Pathol 35: , 1951 Neurol Med Chir (Tokyo) 39, January, 1999

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